Krivosic-Horber R, Adnet P, Krivosic I, Theunynck D, Guévart E, Adamantidis M
Département d'Anesthésie-Réanimation Chirurgicale, Hôpital B, CHR, Lille.
Ann Fr Anesth Reanim. 1988;7(2):132-8. doi: 10.1016/s0750-7658(88)80141-2.
Twenty-seven patients, four of whom had presented with a crisis of malignant hyperthermia and the 23 other being close relatives of such patients, underwent a muscle biopsy so as to determine their susceptibility to malignant hyperthermia. Halothane-caffeine contracture tests, interpreted in accordance with the criteria of the European Group on Malignant Hyperthermia, yielded the following results: 13 positive (MHS), 10 negative (MHN), 4 equivocal (MHE). The history, clinical examination, CPK level, histoenzymatic morphology and electron microscopic study did not provide information sensitive enough to use for the detection of susceptibility to malignant hyperthermia. This confirmed the literature: the halothane-caffeine contracture test remains the only reliable diagnostic test to detect this susceptibility, despite the search for non invasive tests. If the mechanism of triggering a contracture to increasing doses of caffeine is well known in normal muscle, it is the smaller concentrations required which suggests malignant hyperthermia abnormality. The halothane effect is less well understood. A concentration less than or equal to 2 vol % yields a contracture only in MHS muscle. Differences in protocols used by American authors emphasize the importance of standardization as used by the European Group, which is the only way of collecting and comparing results on well over a thousand patients. This confrontation should reduce the number of equivocal diagnoses and allow a correct classification of patients at risk or their relatives as MHS or MHN.
27名患者接受了肌肉活检,以确定他们对恶性高热的易感性,其中4名曾出现恶性高热危象,另外23名是此类患者的近亲。根据欧洲恶性高热研究小组的标准解读氟烷-咖啡因挛缩试验,结果如下:13例阳性(恶性高热易感,MHS),10例阴性(恶性高热非易感,MHN),4例可疑(恶性高热可疑,MHE)。病史、临床检查、肌酸磷酸激酶(CPK)水平、组织酶形态学和电子显微镜研究均未提供足够敏感的信息用于检测恶性高热易感性。这证实了文献观点:尽管一直在寻找非侵入性检测方法,但氟烷-咖啡因挛缩试验仍然是检测这种易感性的唯一可靠诊断试验。如果增加咖啡因剂量引发挛缩的机制在正常肌肉中已为人熟知,那么所需较小浓度则提示存在恶性高热异常。氟烷的作用尚不太清楚。浓度小于或等于2%(体积分数)时,仅在恶性高热易感肌肉中会产生挛缩。美国作者使用的方案差异强调了欧洲研究小组所采用标准化的重要性,这是收集和比较一千多名患者结果的唯一途径。这种对比应能减少可疑诊断的数量,并使对有风险患者或其亲属正确分类为恶性高热易感或非易感成为可能。